Papilledema

Golnaz J avey, M.D.

and
J effrey J . Zuravleff, M.D.

Papilledema specifically refers to optic nerve head swelling secondary to
increased intracranial pressure (IICP). Optic nerve swelling from etiologies other than
IICP is characterized as optic disc edema. Any process which leads to IICP can
likewise cause papilledema. The most common etiologies include tumors of the brain,
spinal cord, intracranial hemorrhage, hydrocephalus, craniosynostosis, cavernous and
dural sinus thrombosis, pseudotumor cerebri (benign intracranial hypertension), head
injury and intracranial infection such as abscess or meningitis.

Most cases of papilledema are bilateral, although the optic nerve appearance can
be asymmetric. Any reported unilateral papilledema should be viewed skeptically and is
more likely optic disc edema. Papilledema is relatively uncommon in infants due to the
lack of fusion of skull bones.

Diagnosis of papilledema is made by direct or indirect ophthalmoscopic
examination of the optic nerves. Hyperemia of the nerve head, blurring of the optic disc
margins, peripapillary flame-shaped hemorrhages, dilation of the capillaries on the
surface of the nerve, and absence of spontaneous venous pulsation are all clinical signs of
papilledema (Figures 1-4) (7).

It must be remembered that not all individuals have
spontaneous venous pulsations so that their absence alone does not indicate IICP. Nerve
fiber layer hemorrhages are the most common finding and often indicate an acute
process. In severe IICP, circumferential retinal folds known as Patons lines may develop
(Figure 4-6) (2, 8)

Chronic papilledema leads to nerve fiber layer atrophy (Figure 6) (6); this
thinning of the nerve fiber bundles, can be best appreciated by examination through the
red- free filter of a direct ophthalmoscope or by slit lamp biomicroscopy. The time
required for papilledema to evolve into optic atrophy depends on severity, and duration of
the IICP.

In chronic papilledema, the compromised blood egress through the central retinal
vein leads to formation of optociliary shunt vessels. These compensatory shunt vessels
indicate the retinal venous blood is bypassing the obstructed retinal vein via the choroidal
venous circulation (4).


Patients with papilledema often complain of headaches, worse upon awakening,
nausea, and vomiting. In early stages of papilledema patients may have no visual
complaints or only be aware of enlargement of the physiologic blind spot. As
papilledema worsens, patients may experience transient obscurations of vision believed
to be secondary to fluctuating ICP, nerve compression and/or or nerve ischemia (3).
Patients with papilledema secondary to an intracranial mass may also have homonymous
visual field defects from lesions in the intracranial visual pathway.
J avey and Zuravleff Page -2- Papilledema


Differential diagnosis of papilledema includes pseudopapilledema (Figure 7)
(anomalous elevated optic disc) and optic nerve edema caused by direct nerve
compression, inflammation, vascular, or infiltrative diseases. The finding of papilledema
in a patient without known intracranial disease, should lead to prompt neuro-imaging and
lumbar puncture.

Treatment of papilledema is directed at the underlying disorder causing IICP.
Papilledema generally resolves 6-8 weeks after ICP is normalized (1). Surgical
intervention is employed when patients present with vision threatening compressive optic
neuropathy or when primary treatment failed to lower the IICP. Cerebrospinal fluid
diversion by ventriculoperitoneal shunt is the most common method for reducing ICP and
optic nerve compression. Optic nerve sheath decompression has been effectively used to
acutely reduce the CSF pressure in the subarachnoid space of the optic nerve; a surgical
window of the nerve sheath allows the CSF to flow into the orbital soft tissues. The
technique is usually employed in severely elevated ICP such as pseudotumor cerebri.
Papilledema may improve in less than a week following optic nerve sheath
decompression surgery (5).

However, the long term effectiveness of this procedure in
reducing IICP is poor.

References:

1. Bettman J W J r, Daroff RB, Sanders MD, et al. Papilledema and symptomatic
intracranial hypertension in systemic lupus erythematosus, Arch Ophthalmol 1968;
80:180-193.

2. Bird AC, Sanders MD. Choroidal folds in association with papilloedema. Br J
Ophthalmol 1973;57:89-97.

3. Cogan DG. Blackouts not obviously due to carotid occlusion. Arch Ophthalmol
1961; 66:180-187.

4. Eggers HM, Sanders MD. Acquired optociliary shunt vessels in papilloedema. Br J
Ophthalmol 1980;64:267-271.

5. Hamed LM, Tse DT, Glaser J S, Frazier Byrne S, Schatz NJ . Neuroimaging of the
optic nerve after fenestration for management of pseudotumor cerebri. Arch Ophthalmol
1992; 110:636-639.

6. Hedges TR III, Legge RH, Peli E, et al. Retinal nerve fiber layer changes and visual
field loss in idiopathic intracranial hypertension. Ophthalmology 1995; 102: 1242-1247.

7. Neetens A, Smets RM. Papilledema. Neuroophthalmology 1989; 9:81-101.

8. Nettleship E. Peculiar lines in the choroid in a case of post-papillitic atrophy. Trans
Ophthalmol Soc UK 1884; 4: 167-8.

Figure 1. Courtesy of Mr. David B. Bennett, CRA.
Papilledema with blurring of disc margins.



Figure 2. Courtesy of Mr. David B. Bennett, CRA.
Papilledema with blurring of disc margins and dilation of surface capillaries.


Figures 3. Courtesy of Mr. David B. Bennett, CRA.
Papilledema with blurring of disc margins, disc hyperemia, and peripapillary flame
shaped hemorrhages.



Figure 4. Courtesy of Mr. David B. Bennett, CRA.
Papilledema with peripapillary flame shaped hemorrhages. Note the
circumferential retinal folds also known as Patons lines.



Figure 5. Courtesy of Mr. David B. Bennett, CRA
Papilledema with blurry disc margins and Patons lines.



Figure 6. Courtesy of Mr. David B. Bennett, CRA
Chronic papilledema with neurofibrolayer atrophy and Patons lines.
Note the extensive retinal exudates in the maculo-papillary bundle.


Figure 7. Courtesy of Mr. David B. Bennett, CRA
Pseudopapilledema. Optic nerve head drusen causing elevation and blurring of the
optic nerve head.